{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:28:02Z","timestamp":1791300482387,"version":"4.1.0"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2017,6,27]],"date-time":"2017-06-27T00:00:00Z","timestamp":1498521600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000066","name":"National Institute of Environmental Health Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000066","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Autoantibodies recognizing 3\u2010hydroxy\u20103\u2010methylglutaryl\u2010coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of juvenile anti\u2010HMGCR\u2010positive myositis patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The sera of 440 juvenile myositis patients were screened for anti\u2010HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti\u2010HMGCR\u2010positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis\u2010specific autoantibodies (MSAs).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Five of 440 patients (1.1%) were anti\u2010HMGCR\u2010positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and 2 patients had immune\u2010mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti\u2010HMGCR\u2010positive subjects was 17,000 IU\/liter. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR\u2010positive subjects compared to MSA\u2010negative patients or those with other MSAs. Anti\u2010HMGCR\u2010positive patients had only partial responses to multiple immunosuppressive medications, and their disease often took a chronic course. The DRB1*07:01 allele was present in all 5 patients, compared to 26.25% of healthy controls (corrected <jats:italic>P<\/jats:italic>\u2009=\u20090.01); none of the 5 juvenile patients had DRB1*11:01.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Compared to children with other MSAs, muscle disease appears to be more severe in those with anti\u2010HMGCR autoantibodies. Like adults, children with anti\u2010HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, in anti\u2010HMGCR\u2010positive children, there is a strong association with HLA\u2013DRB1*07:01.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23113","type":"journal-article","created":{"date-parts":[[2017,1,27]],"date-time":"2017-01-27T12:27:06Z","timestamp":1485520026000},"page":"1088-1094","source":"Crossref","is-referenced-by-count":101,"title":["Association of Anti\u20133\u2010Hydroxy\u20103\u2010Methylglutaryl\u2010Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients"],"prefix":"10.1002","volume":"69","author":[{"given":"Takayuki","family":"Kishi","sequence":"first","affiliation":[{"name":"National Institute of Environmental Health Sciences, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa G.","family":"Rider","sequence":"additional","affiliation":[{"name":"National Institute of Environmental Health Sciences, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Pak","sequence":"additional","affiliation":[{"name":"Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilliana","family":"Barillas\u2010Arias","sequence":"additional","affiliation":[{"name":"Albany Medical Center Albany New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Henrickson","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Medical Center Cincinnati OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul L.","family":"McCarthy","sequence":"additional","affiliation":[{"name":"Yale University School of Medicine New Haven Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bracha","family":"Shaham","sequence":"additional","affiliation":[{"name":"Children's Hospital of Los Angeles Los Angeles California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela F.","family":"Weiss","sequence":"additional","affiliation":[{"name":"Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iren","family":"Horkayne\u2010Szakaly","sequence":"additional","affiliation":[{"name":"Joint Pathology Center, Defense Health Agency Silver Spring Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ira N.","family":"Targoff","sequence":"additional","affiliation":[{"name":"Veterans Affairs Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation Oklahoma City Oklahoma."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederick W.","family":"Miller","sequence":"additional","affiliation":[{"name":"National Institute of Environmental Health Sciences, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew L.","family":"Mammen","sequence":"additional","affiliation":[{"name":"Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the Childhood Myositis Heterogeneity Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,27]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2010.1977"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kem359"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30156"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2016-313166"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24541"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kev229"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0141616"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000000028"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e31827f264d"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39466"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22216"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21671"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21291"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0277-y"}],"container-title":["Arthritis Care &amp; 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